Low Origin of the Coronary Arteries and a Small Aortic Annulus Complicating Aortic Valve Replacement.

Low origin of the coronary arteries, defined as an origin less than 10 mm above the functional aortic annulus, is not usually considered to be a notable anomaly because functional impairment is not intrinsic. We describe a case of severe complications after surgical aortic valve replacement in a 59-year-old woman who had symptomatic aortic valve stenosis, low origin of both main coronary arteries, and a hypoplastic aortic annulus less than 19 mm in diameter. The aortic prosthesis had to be implanted above the hypoplastic anatomic annulus. An inferior-wall myocardial infarction, hypotension, right-sided heart failure, and atrial fibrillation developed during the early perioperative period. Coronary angiograms showed occlusion of the right coronary artery ostium and critical stenosis of the left coronary ostium. During reoperation, posterior aortic patch annuloplasty enabled lower reimplantation of the prosthetic aortic valve, jointly with right coronary artery-venous grafting. To prevent potentially severe complications, we recommend that low origin of the coronary arteries be reported before patients undergo surgical aortic valve replacement. If the ostia are not seen when routine coronary angiography is used, computed tomography should be prospectively performed to characterize this anomaly.

Is there a delay in seeking medical care after the first seizure in "resource limited settings": a pilot study from Sri Lanka.

OBJECTIVES: Current guidelines suggest that patients presenting with the first seizure should be assessed by a specialist, preferably with investigations such as electroencephalography and imaging to reach a definitive diagnosis. We conducted a cross sectional study among patients with confirmed epilepsy, at a tertiary level neurology clinic in Sri Lanka with the aim of assessing delays in first contact with a medical doctor and in performing key investigations after the first seizure. RESULTS: Majority had sought medical attention within 24 h of the first seizure (71.2%) and had seen a specialist within the 1st week since the seizure (61%). Also a significant proportion had completed key investigations such as electroencephalography (63.2%) and brain imaging within a month (51%) since the first medical consultation. Of many socio-demographic and illness related factors examined, only a non-generalized tonic-clonic presentation was significantly associated with delay in seeking medical help.

Evolution into Takayasu arteritis in a patient presenting with acute pulmonary oedema due to severe aortic regurgitation; a case report.

BACKGROUND: Takayasu arteritis is a rare large vessel vasculitis which predominantly affects young Asian females. Aortic regurgitation and heart failure are well described manifestations which are usually preceded by constitutional symptoms, limb claudication, pulse and blood pressure discrepancies, vascular bruits and features of organ ischaemia. CASE PRESENTATION: A 25-year- old Sri Lankan female presented with a three days history of acute shortness of breath, cough and orthopnoea. On examination she had severe aortic regurgitation resulting in high output cardiac failure. There was no evidence of acute coronary ischaemia or infective endocarditis. The only significant investigation finding was an elevated erythrocyte sedimentation rate (ESR) of 114 mm/first hour. The patient was treated for pulmonary oedema and empirically for infective endocarditis. Extensive evaluation for an underlying infection, large vessel vasculitis or malignancy did not reveal any abnormalities. Detailed periodic assessment identified reduced blood pressure in left arm (70/40 mmHg) compared to right (100/70 mmHg) and reduced pulse volume of left arm with left subclavian bruit more than one year after the initial presentation. Digital subtraction angiography revealed significant stenosis at first part of left subclavian and origin of left vertebral arteries. A diagnosis of Takayasu arteritis was made and patient was started on high dose glucocorticoids. CONCLUSIONS: Takayasu arteritis can present initially with isolated cardiac involvement even as acute cardiac manifestations and high degree of suspicion with close follow up would allow early detection of development of other classic features and timely diagnosis.

Takotsubo cardiomyopathy complicated with apical thrombus formation on first day of the illness: a case report and literature review.

BACKGROUND: Takotsubo cardiomyopathy is characterized by transient systolic dysfunction of the apical and mid segments of the left ventricle in the absence of obstructive coronary artery disease. Intraventricular thrombus formation is a rare complication of Takotsubo cardiomyopathy and current data almost exclusively consists of isolated case reports and a few case series. Here we describe a case of Takotsubo cardiomyopathy with formation of an apical thrombus within 24 h of symptom onset, which has been reported in the literature only once previously, to the best of our knowledge. We have reviewed the available literature that may aid clinicians in their approach to the condition, since no published guidelines are available. CASE PRESENTATION: A 68-year-old Sri Lankan female presented to a local hospital with chest pain. Electrocardiogram (ECG) showed ST elevation, and antiplatelets, intravenous streptokinase and a high dose statin were administered. Despite this ST elevation persisted; however the coronary angiogram was negative for obstructive coronary artery disease. Echocardiogram revealed hypokinesia of the mid and apical segments of the left ventricle with typical apical ballooning and a sizable apical thrombus. She had recently had a viral infection and was also emotionally distressed as her sister was recently diagnosed with a terminal cancer. A diagnosis of Takotsubo cardiomyopathy was made and anticoagulation was started with heparin and warfarin. The follow up echocardiogram performed 1 week later revealed a small persistent thrombus, which had completely resolved at 3 weeks. CONCLUSION: Though severe systolic dysfunction is observed in almost all the patients with Takotsubo cardiomyopathy, intraventricular thrombus formation on the first day of the illness is rare. The possibility of underdiagnosis of thrombus can be prevented by early echocardiogram in Takotsubo cardiomyopathy. The majority of reports found in the literature review were of cases that had formed an intraventriclar thrombus within the first 2 weeks, emphasizing the importance of follow up echocardiography at least 2 weeks later. The management of a left ventricular thrombus in Takotsubo cardiomyopathy is controversial and in most cases warfarin and heparin were used for a short duration.

Prevalence and associations for symptoms of depression in patients with Parkinson's disease: a Sri Lankan experience.

BACKGROUND: The prevalence and associations for depression in patients with Parkinson's disease vary widely between studies. This reflects the influence of cultural, demographic and socioeconomic confounders within communities that make generalizations invalid. Therefore it is important to identify unique attributes within a community on this phenomenon. This is the first study from Sri Lanka on the prevalence and associations for co-morbid depression in patients with Parkinson's disease. METHODS: We conducted this cross sectional study at the Institute of Neurology, National Hospital of Sri Lanka. All patients with a diagnosis of idiopathic Parkinson's disease followed up at the movement disorder clinic of the institute were enrolled. The patients were interviewed by investigators (medical practitioners) with an interviewer administered questionnaire that collected data on (a) demography, (b) clinical symptoms of Parkinson's disease and (c) socioeconomic background. Symptoms of depression were assessed with Hamilton rating scale for depression. RESULTS: We enrolled 75 patients [males; 54 (75 %), mean age; 63.6 years, SD ± 6.8]. Forty-six (61.3 %) patients had been either formally diagnosed with depression or showed symptoms of depression. Bradykinesia, monthly income below Rs. 10,000 and having a family history of depression were significantly associated with a diagnosis of life-time post Parkinson's disease depression (p < 0.05). CONCLUSION: Given the potential benefit in treatment (for depression), all patients with Parkinson's disease should be screened for depression regularly. HAM-D would be a good screening tool for this purpose as it has good reliability, validity and can be administered within a reasonable time limit.

Knowledge seeking behaviours of pre interns and early career doctors in Sri Lanka: a cross sectional study.

BACKGROUND: Use of reference sources for medical knowledge has changed dramatically over the last two decades with the introduction of online sources of information. This study analyses the medical knowledge seeking behaviours of pre interns and early career doctors in Sri Lanka. METHODS: This cross sectional survey with a convenience sample was conducted at two sites targeting two groups; pre-intern doctors graduated from the Faculty of Medicine, University of Colombo and early career doctors following a postgraduate course at the National Hospital of Sri Lanka. The data collection tool was an online self-administered questionnaire (paper based questionnaires used on request) that probed the patterns of using reference sources for medical knowledge. RESULTS AND DISCUSSION: The respondents comprised of 52 pre-interns and 34 early career doctors. A majority (98 %) had internet access. Early career doctors preferred online resources significantly more than the pre-interns. However, the utilization of online resources for evidence synthesis and planning research was unsatisfactory in both groups. A significant proportion (35 %) responded that they had never read a systematic review. Only one person in the entire sample had co-authored a review article. CONCLUSION: The use of online resources by participants seems to be satisfactory with a majority shifting to reliable online resources as a reference point for medical knowledge. However, a closer look at the usage patterns reveal that online resources that can be used for more innovative tasks such as evidence synthesis are grossly under-utilized.

Gaze palsy, hypogeusia and a probable association with miscarriage of pregnancy--the expanding clinical spectrum of non-opticospinal neuromyelitis optica spectrum disorders: a case report.

BACKGROUND: Neuromyelitis optica is characterised by optic neuritis, longitudinally-extensive transverse myelitis and presence of anti-aquaporin-4 antibodies in the serum. However, non-opticospinal central nervous system manifestations have been increasingly recognised. Awareness of the widening clinical spectrum of neuromyelitis optica (unified within the nosology of 'neuromyelitis optica spectrum disorders') is key to earlier diagnosis and appropriate therapy. We report 2 patients to illustrate the varied clinical manifestations of neuromyelitis optica spectrum disorders while postulating an effect of anti-aquaporin-4 antibodies on the miscarriage of pregnancy. This is the first report of horizontal gaze palsy as a presenting symptom of neuromyelitis optica spectrum disorders. CASE PRESENTATION: Patient 1: A 17-year-old Sri Lankan female presented with hypersomnolence, lateral gaze palsy and loss of taste of 1 week duration. Two years previously she had presented with intractable hiccups and vomiting followed by a brainstem syndrome. Magnetic resonance imaging showed a lesion in the left cerebellum extending into the pons while lesions in bilateral hypothalami and medulla noted 2 years ago had resolved. Autoimmune, vasculitis and infection screens were negative. Anti-aquaporin-4 antibodies were detected in serum. All her symptoms resolved with immunosuppressive therapy. Patient 2: A 47-Year-old Sri Lankan female presented with persistent vomiting lasting over 3 weeks. Three years previously, at 25-weeks of her 4(th) pregnancy, she had presented with quadriparesis and was found to have a longitudinally extensive transverse myelitis from C2 to T2 vertebral levels, which gradually improved following intravenous steroid therapy. Magnetic resonance imaging showed a hyper-intense lesion in the area postrema and longitudinally extensive atrophy of the cord corresponding to her previous myelitis. Autoimmune, vasculitis and infection screens were negative. Anti-aquaporin-4 antibodies were detected in serum. Her vomiting subsided with immunosuppressive therapy. Her second pregnancy had resulted in a first-trimester miscarriage. CONCLUSION: The clinical spectrum of neuromyelitis optica spectrum disorders has expanded beyond optic neuritis and myelitis to include non-opticospinal syndromes involving the diencephalon, brainstem and cerebrum. Our report highlights the varied central nervous system manifestations of neuromyelitis optica spectrum disorders and miscarriage of pregnancy possibly related to anti-aquaporin-4 antibodies.

Cyclophosphamide-induced posterior reversible encephalopathy syndrome (PRES): a case report.

INTRODUCTION: Posterior reversible encephalopathy syndrome is a clinicoradiologic entity characterized by headache, seizures, decreased vision, impaired consciousness and white matter oedema in bilateral occipitoparietal regions. Hypertensive encephalopathy, eclampsia, immunosuppressive/cytotoxic drugs, organ transplantation, renal disease, autoimmune diseases and vasculitides are reported risk factors of posterior reversible encephalopathy syndrome. Reports of cyclophosphamide-induced posterior reversible encephalopathy syndrome are rare and occurred in a background of renal failure, fluid overload or active connective tissue disease. CASE PRESENTATION: We report a case of posterior reversible encephalopathy syndrome developing as a direct consequence of intravenous cyclophosphamide therapy in a 33-year-old normotensive Sri Lankan woman with lupus nephritis but quiescent disease activity and normal renal function. CONCLUSIONS: This case report highlights the need for awareness and early recognition of this rare but serious adverse effect of cyclophosphamide that occurred in the absence of other known risk factors of posterior reversible encephalopathy syndrome and that early appropriate intervention leads to a good outcome.

Klippel-Trenaunay syndrome presenting with acanthocytosis and splenic and retroperitoneal lymphangioma: a case report.

INTRODUCTION: Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by bone and soft tissue hypertrophy, extensive hemangioma and venous abnormalities. We report the case of a patient with two additional rare clinical manifestations in the background of Klippel-Trenaunay syndrome, namely, acanthocytosis and splenic and retroperitoneal lymphangioma. CASE PRESENTATION: A 24-year-old Sri Lankan man from North Central Province in Sri Lanka presented to our general medical unit with symptomatic anaemia. He had been diagnosed with Klippel-Trenaunay syndrome at the age of six years, with hemihypertrophy of his right lower limb and strawberry naevi over both lower limbs. His blood film results were positive for acanthocytes, which accounted for more than 20% of the red blood cell population. He was also found to have extensive splenic lymphangiomas and a large retroperitoneal lymphangioma encasing the mesentric vessels in the right para-aortic region. An extensive battery of tests to identify a secondary cause for the acanthocytosis failed to show any positive results. CONCLUSIONS: Retroperitoneal lymphangioma has been reported in association with Klippel-Trenaunay syndrome once before, but an association with acanthocytosis has never been reported. Given the rarity of all three conditions this is not surprising. The cause of acanthocytosis in this setting is currently unresolved. It is plausible that this may be a primary association with Klippel-Trenaunay syndrome, as an alternative aetiology was not found.

Presumptive thrombotic thrombocytopenic purpura following a hump-nosed viper (Hypnale hypnale) bite: a case report.

Hump-nosed viper bites are frequent in southern India and Sri Lanka. However, the published literature on this snakebite is limited and its venom composition is not well characterized. In this case, we report a patient with thrombotic thrombocytopenic purpura-like syndrome following envenoming which, to the best of our knowledge, has not been reported in the literature before. A 55-year-old woman from southern Sri Lanka presented to the local hospital 12 hours after a hump-nosed viper (Hypnale hypnale) bite. Five days later, she developed a syndrome that was characteristic of thrombotic thrombocytopenic purpura with fever, thrombocytopenia, microangiopathic hemolysis, renal impairment and neurological dysfunction in the form of confusion and coma. Her clinical syndrome and relevant laboratory parameters improved after she was treated with therapeutic plasma exchange. We compared our observations on this patient with the current literature and concluded that thrombotic thrombocytopenic purpura is a theoretically plausible yet unreported manifestation of hump-nosed viper bite up to this moment. This study also provides an important message for clinicians to look out for this complication in hump-nosed viper bites since timely treatment can be lifesaving.

Dengue fever presenting with acute cerebellitis: a case report.

BACKGROUND: The incidence of dengue fever is on the rise in tropical countries. In Sri Lanka, nearly 45,000 patients were reported in 2012. With the increasing numbers, rare manifestations of dengue are occasionally encountered. We report a patient who presented with bilateral cerebellar signs as the presenting feature of dengue. CASE PRESENTATION: A 45-year-old previously healthy female from the suburbs of Colombo, Sri Lanka presented with an acute febrile illness associated with unsteadiness of gait. Clinical examination revealed a scanning dysarthria and marked horizontal nystagmus with bilateral dysmetria, dysdiadokokinesia and incordination more prominent on the right. Her gait was wide-based and ataxic with a tendency to fall to the right more than to the left. Dengue nonstructural protein antigen 1 test and IgM antibody testing both became positive indicating acute dengue infection. She recovered from the febrile episode within 9 days since the onset of fever but cerebellar symptoms outlasted the fever by one week. The magnetic resonance imaging of brain was normal and cerebellar signs resolved spontaneously by day 17 of the illness. CONCLUSIONS: Cerebellar syndrome in association with dengue fever has been reported in only four instances and our patient is the first reported case of dengue fever presenting with cerebellitis as the first manifestation of disease. This case report is intended to highlight the occurrence of acute cerebellitis as a presenting syndrome of the expanding list of unusual neurological manifestations of dengue infection.

Presumptive thrombotic thrombocytopenic purpura following a hump-nosed viper (Hypnale hypnale) bite: a case report

Hump-nosed viper bites are frequent in southern India and Sri Lanka. However, the published literature on this snakebite is limited and its venom composition is not well characterized. In this case, we report a patient with thrombotic thrombocytopenic purpura-like syndrome following envenoming which, to the best of our knowledge, has not been reported in the literature before. A 55-year-old woman from southern Sri Lanka presented to the local hospital 12 hours after a hump-nosed viper (Hypnale hypnale) bite. Five days later, she developed a syndrome that was characteristic of thrombotic thrombocytopenic purpura with fever, thrombocytopenia, microangiopathic hemolysis, renal impairment and neurological dysfunction in the form of confusion and coma. Her clinical syndrome and relevant laboratory parameters improved after she was treated with therapeutic plasma exchange. We compared our observations on this patient with the current literature and concluded that thrombotic thrombocytopenic purpura is a theoretically plausible yet unreported manifestation of hump-nosed viper bite up to this moment. This study also provides an important message for clinicians to look out for this complication in hump-nosed viper bites since timely treatment can be lifesaving.

Presumptive thrombotic thrombocytopenic purpura following a hump-nosed viper (Hypnale hypnale) bite: a case report

Hump-nosed viper bites are frequent in southern India and Sri Lanka. However, the published literature on this snakebite is limited and its venom composition is not well characterized. In this case, we report a patient with thrombotic thrombocytopenic purpura-like syndrome following envenoming which, to the best of our knowledge, has not been reported in the literature before. A 55-year-old woman from southern Sri Lanka presented to the local hospital 12 hours after a hump-nosed viper (Hypnale hypnale) bite. Five days later, she developed a syndrome that was characteristic of thrombotic thrombocytopenic purpura with fever, thrombocytopenia, microangiopathic hemolysis, renal impairment and neurological dysfunction in the form of confusion and coma. Her clinical syndrome and relevant laboratory parameters improved after she was treated with therapeutic plasma exchange. We compared our observations on this patient with the current literature and concluded that thrombotic thrombocytopenic purpura is a theoretically plausible yet unreported manifestation of hump-nosed viper bite up to this moment. This study also provides an important message for clinicians to look out for this complication in hump-nosed viper bites since timely treatment can be lifesaving.